Neurofibromatosis (NF) is a genetically inherited, autosomal dominant disease, characterized by multiple cafe au lait spots, cutaneous neurofibromas and "Lisch nodules. " Neurofibromatosis can develop from a neural source at any age. However, neurofibroma of the larynx is extremely rare and is usually manifested by obstructive airway symptoms. We encountered a 5-year-old child presenting with stridor and dyspnea, who had a diagnosis of laryngeal plexiform neurofibroma. The purpose of our report is the consideration of laryngeal NF in the differential diagnosis of dyspnea in infants and children.